Progressive Airway Obstruction due to Plexiform Neurofibromain 7 Years-Old Female

PutuDiah Pratiwi | I.B Subanada | NP. Siadi Purniti | Ayu Setyorini ^[2] | I GdeArdika Nuaba

Abstract: Background: Extention to larynx of Plexiform neurofibromas are extremely rare, approximately for only 0, 03 to 0, 1 % of benign tumors of the larynx, it is one of neurofibroma type 1 (NF-1) variant.1, 6 Surgery is the only choice to achieve temporary tumor growth control and symptom progression. Patient with small plexiform neurofibroma eligible for complete resection of tumor. Case: 7 years old female presented with dyspnea. Physical examination showed a lot of subcutaneous neurofibroma. Endoscopy shown massive airway obstruction, that was narrowed by greater than 50 % than decide emergency tracheostomy. Unfortunately, the patient getting worse because of progressive airway obstruction lower the site of tracheostomy and patient death because airway obstruction Learning Points: Tumors location in head/neck/face cannot be completely removed, this tumor had been reported difficult to separate from normal tissue because they lack a well-defined capsule. Airway compression and spontaneous bleeding are direct lethal complication of plexiform neurofibroma.

Keywords: neurofibroma, plexiform neurofibroma, airway obstruction

Edition: Volume 8 Issue 2, February 2019,

Pages: 2289 - 2292